Lupus: Everything You Need to Know About the Disease, What Causes It, and How to Treat It

Lupus is an autoimmune disease — meaning the body’s own immune system doesn’t function properly and attacks you by mistake — and affects multiple organs throughout the body. In healthy individuals, the body produces proteins called antibodies to fight off foreign invaders such as viruses, bacteria, and fungi.

But in people with lupus, antibodies can’t distinguish between foreign invaders and the body’s own cells and tissues. These autoantibodies attack cells and tissues, causing inflammation, which can lead to arthritis, lupus rash, kidney damage, and other symptoms and health problems. (1)

Lupus is “the epitome of an autoimmune disease,” says Stuart D. Kaplan, MD, the chief of rheumatology at South Nassau Communities Hospital in Oceanside, New York. “It’s the body fighting itself and making antibodies against one’s own cells.”

This lifelong, chronic disease has the potential to affect almost every organ system in the body, although not all systems are affected in any one person. The first thing patients need to know is that lupus varies in symptoms and severity from person to person, adds Dr. Kaplan. Some people have active disease while other have mild disease.

Here are some other important facts to know:

Lupus is diagnosed in about 16,000 people each year. (2) Overall, an estimated 322,000 to 1.5 million Americans have the disease. (3)

Lupus is NOT contagious.

Lupus is not necessarily life threatening when treated appropriately. Up to 90 percent of patients will have a normal life expectancy if they are followed closely by their doctor and receive proper treatment. (4,5) Lupus can, however, increase mortality rates because patients have a higher risk of heart disease, infection or complications such as inflammation of the kidney, or nephritis, says Francis Luk, MD, an assistant professor of rheumatology and immunology, Wake Forest Baptist Medical Center.

More on Lupus

The Most Common Type of Lupus Is Systemic Lupus Erythematosus (SLE)

There are several types of lupus:

Systemic lupus erythematosus (SLE) About 70 percent of people diagnosed with lupus have this form, making it the most common. In some people, the disease is mild and may cause only rash and some joint pain, says Kaplan. In others, inflammation can lead to inflammation of the kidney (also known as lupus nephritis) or other complications, he says.

Cutaneous lupus erythematosus This form of lupus develops in the skin as a rash. The most common form of cutaneous lupus erythematosus is discoid — which refers to the appearance of round, raised, red, and scaly rash that does not itch. (6) About 10 percent of discoid lupus patients can go on to develop SLE.

Drug-induced lupus erythematosus (DIL) Some drugs can cause lupus, resulting in symptoms such as rash, arthritis, hair loss, and fever. “Once medications are discontinued, the symptoms go away,” says Roberto Caricchio, MD, the interim section chief of rheumatology at Temple University Hospital in Philadelphia and the director of the Temple Lupus Clinic at the Lewis Katz School of Medicine.

Neonatal lupus Technically neonatal lupus is not a form of lupus. The condition is the result of autoantibodies passing from a pregnant woman with lupus (or related condition) through the placenta and to the baby developing in the womb, causing mostly temporary symptoms, explains Virginia Pascual, MD, the director of the Gale and Ira Drukier Institute for Children’s Health at Weill Cornell Medicine in New York City. Some infants are born with symptoms, such as skin rash, liver problems, or white blood cell counts. But those symptoms disappear within a few months and leave no lasting effects.

Lupus Symptoms: All About the Rash, Flares, and Complications

“The vast majority of lupus patients at some point will have joint pain with inflammation, some form of skin rash, and fatigue,” says Dr. Caricchio.Other common symptoms can include: (7,8)

Fever

Headaches

Sun sensitivity

Low red blood cell counts (anemia)

Swelling of the hands, arms, feet, legs, and around the eyes (edema)

Mouth or nose sores

Hair loss

Blood clotting

Raynaud's phenomenon, in which the small blood vessels in the fingers and toes spasm, limiting circulation, especially in cold weather

It’s important to note that cases of lupus, symptoms, and symptom severity differ from person to person, and new symptoms can appear at any time.

Most people don’t experience symptoms continuously. Instead, there are different times when symptoms appear, known as lupus flares. How long flares last varies from patient to patient, but symptoms can typically persist from a few days to weeks at a time. “There’s no way to predict when a flare will occur,” says Dr. Luk.

Flare symptoms can also be wide ranging. Some people may experience relatively mild flares that include rash and arthritis, while others have severe flares such as kidney inflammation, adds Luk.

Stress may contribute to the immune system not functioning properly, explains Kaplan. And both emotional and physical stress, such as from surgery, have been known to exacerbate lupus.

Many women with lupus find that the condition may flare before their period. Estrogen peaks around day 14 of the menstrual cycle, but “it takes a while for the flare to develop,” says Kaplan. Lupus can also flare during or shortly after pregnancy, he adds.

Complications of lupus can include:

Nephritis, or inflammation of the kidney, which can be mild or severe

Fluid around the heart and the lungs, or pleural effusion, which can cause shortness of breath or chest pain

Blockages of the blood vessels leading to the brain, causing stroke

Inflammation of the heart, or myocarditis, which can cause chest pain and heart disease

Inflammation of the brain, which can cause confusion, seizures, and psychosis

How You Get Lupus: Genetics and Other Factors Play a Role

More on Lupus Causes

The answer to what causes lupus is “we do not know,” says Caricchio. Research has yet to pinpoint the exact conditions or triggers that lead to the disease, he explains — “however the condition appears to be multifactorial."

Some of those factors that experts suspect play a role are:

Genetics Doctors and researchers believe a genetic predisposition may contribute to the development of lupus, says Kaplan. Dozens of genetic variations have been found to be associated with the disease, affecting who gets it and how severe those cases are, according to the Lupus Foundation of America. That means the disease is hereditary, making parents more likely to pass it to their children. But just because you are genetically predisposed to the condition, doesn’t necessarily mean you’ll get it.

Environment Researchers suspect environmental factors may increase the risk of developing lupus. For example, exposure to sun can cause a lupus rash and some systemic lupus activity, says Stacy Ardoin, MD, a rheumatologist at the Ohio State University Wexner Medical Center in Columbus. Other environmental factors that may contribute to lupus can include some drugs, viral infections, exhaustion, stress, and anything that can cause physical stress to the body (such as surgery, physical harm, injury, pregnancy, or giving birth).

Hormones Lupus is much more common in women than in men. One reason for this may be because of higher estrogen levels in women, notes Kaplan — though that has not yet been proved in scientific studies. (9)

How Lupus Gets Diagnosed and Tests Your Doctor May Order

Diagnosing lupus “can be very challenging,” Ardoin says. This is because symptoms can range from a mild rash and arthritis to kidney failure and seizures — “with a whole spectrum in between.” Symptoms may mimic other diseases and conditions, such as infections or cancer.

To diagnose lupus, doctors typically conduct blood work to look for antibodies associated with the disease. For example, antinuclear antibodies bind to cell nuclei, damaging the cells or causing them to die. About 97 percent of people with lupus have these antibodies. (10)

Other tests for lupus depend on the symptoms patients are experiencing, says Kaplan. For example, chest X-rays and echocardiograms may be necessary to investigate fluid around the lungs and the heart. If doctors suspect nephritis is present, the patient may need a kidney biopsy. Early diagnosis and treatment can help to avoid complications, he adds.

An accurate diagnosis of lupus can take time, says Caricchio. However, the availability of good lupus classification criteria can make the chance of receiving a wrong diagnosis slim, he adds. Classification criteria are a set of symptoms doctors use after they suspect that you have lupus, to determine the type and severity of your case.

Doctors use two different systems to diagnose the condition: American College of Rheumatologycriteria (11), published in 1997, and anewer system, Systemic Lupus International Collaborating Clinics Classification criteria for systemic lupus erythematosus, or SLICC. (12)

Fortunately, increased awareness among the general public and primary care physicians may mean patients may see a rheumatologist sooner rather than later for diagnosis, says Caricchio.

Other drugs used to treat lupus include the antimalarial drug hydroxychloroquine, which modulates the immune system, and belimumab, a targeted drug that is a biologic (meaning it’s made from natural sources). Some chemotherapy drugs and anti-rejection drugs may be used, too, to treat patients with lupus nephritis or other organ problems, says Caricchio.

Why Lupus Monitoring Is Important

While lupus is more treatable than it used to be, the disease can still be life-threatening, Ardoin notes. “It’s hard to predict the course of illness, so it’s important for patients to check in regularly with their rheumatologist,” she says.

After people are diagnosed and begin treatment, they need to see their rheumatologists regularly for blood draws to monitor white blood cells, red blood cells, and platelet levels, says Ardoin.

Blood tests also help doctors monitor levels of complement proteins, which boost the body’s immune response to infections. (Levels of complement proteins, as well as some others, are low when the disease is active, explains Ardoin.)

Blood and urine tests are also needed to check kidney function. “It’s important to regularly follow up with a rheumatologist to make sure the disease is not starting to become more severe,” says Luk. If kidney problems are not detected early, the risk of renal failure and death are higher.

“It’s hard to predict the course of illness, so it’s important for patients [with lupus] to check in regularly with their rheumatologist."

- Stacy Ardoin, MD, a rheumatologist at the Ohio State University Wexner Medical Center in Columbus

Lupus Doesn’t Need to Hold You Back or Stop You From Living a Normal Life

While a lupus diagnosis can be overwhelming, if the disease is controlled, people living with the condition should be able to “go about life as normally as people who don’t have lupus,” Luk says.

Individuals should go on pursuing activities or hobbies they enjoyed before diagnosis, adds Caricchio. And, he says: Women with lupus can have children. “We don’t discourage having kids by any means,” he says. “But it is recommended that women become pregnant when lupus is not active, so that the chance of flare is minimal.”

Because lupus is associated with an increased risk of cardiovascular disease, whether due to inflammation or genetics, maintaining a healthy lifestyle is particularly important, says Caricchio. He recommends getting enough sleep, maintaining a healthy body weight, exercising, and eating a nutritious diet.

Additionally, people with lupus should be mindful to avoid sun exposure as much as possible by wearing sunscreen and protective clothing, says Kaplan. A photosensitive rash can become worse in UV light.

Good social relationships and support groups can also be important for maintaining mental health, says Ardoin.

Notably, several celebrities with lupus have demonstrated that having the disease doesn’t mean forfeiting life goals and interests. For example, the singer and actress Selena Gomez has undergone chemotherapy to treat the disease and received a kidney transplant to address lupus nephritis. (13)

When Lupus Affects Children: Testing, Treatment, and Living With It

Lupus in children tends to be more aggressive than in adults, says Dr. Pascual. The exact reasons for this are not understood. One theory is that people are born with genetic susceptibility to the disease that may be triggered by environmental factors such as a virus. “Children with the condition may have inherited a more complex set of predisposing genes,” she says. But this theory has yet to be proved.

Children undergo the same diagnostic testing as adults and treatments are similar. “There are few clinical trials in children, so there is no option but to treat children based on the adult experience. We use the same drugs,” says Pascual, with doses adjusted according to the child's weight.

Doctors should closely monitor children for drug side effects. Steroids, for example, can delay growth and cause high blood pressure. Chemotherapy can make children more prone to infections, says Pascual.

“It’s always difficult for children and parents to live with the idea that lupus is chronic,” says Pascual. That means the child has many more years worth of living with the condition than if he or she were diagnosed later in life. And because this disease is lifelong and may involve complications such as nephritis, doctors need to manage it aggressively.

But fortunately, life expectancy in pediatric lupus has improved dramatically in the last 15 years, she says.